Adenovirus-mediated in vivo gene transfer rapidly protects ornithine transcarbamylase-deficient mice from an ammonium challenge.

Adenovirus-mediated in vivo gene transfer rapidly protects ornithine transcarbamylase-deficient mice from an ammonium challenge.

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http://www.wikidata.org/entity/Q45881297
Ammonia triggers neuronal disinhibition and seizures by impairing astrocyte potassium buffering.Preclinical evaluation of a clinical candidate AAV8 vector for ornithine transcarbamylase (OTC) deficiency reveals functional enzyme from each persisting vector genome.Sustained correction of OTC deficiency in spf( ash) mice using optimized self-complementary AAV2/8 vectors.A novel biochemically salvageable animal model of hyperammonemia devoid of N-acetylglutamate synthaseHelper-dependent adenoviral vectors in experimental gene therapy.SCN4A pore mutation pathogenetically contributes to autosomal dominant essential tremor and may increase susceptibility to epilepsy.AAV-based gene therapy prevents neuropathology and results in normal cognitive development in the hyperargininemic mouse.Overexpression of the cholesterol-binding protein MLN64 induces liver damage in the mouse.Correction of argininosuccinate synthetase (AS) deficiency in a murine model of citrullinemia with recombinant adenovirus carrying human AS cDNA.Enhancement of hepatic autophagy increases ureagenesis and protects against hyperammonemia.Down-regulation of hepatocyte nuclear factor-4α and defective zonation in livers expressing mutant Z α1-antitrypsin.
P2860

Adenovirus-mediated in vivo gene transfer rapidly protects ornithine transcarbamylase-deficient mice from an ammonium challenge.

Item
http://www.wikidata.org/entity/Q45881297
description
1997 nî lūn-bûn
@nan
1997年の論文
@ja
1997年学术文章
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1997年学术文章
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1997年学术文章
@zh-hans
1997年学术文章
@zh-my
1997年学术文章
@zh-sg
1997年學術文章
@yue
1997年學術文章
@zh
1997年學術文章
@zh-hant
name
Adenovirus-mediated in vivo ge ...... ce from an ammonium challenge.
@en
Adenovirus-mediated in vivo ge ...... ce from an ammonium challenge.
@nl
type
label
Adenovirus-mediated in vivo ge ...... ce from an ammonium challenge.
@en
Adenovirus-mediated in vivo ge ...... ce from an ammonium challenge.
@nl
prefLabel
Adenovirus-mediated in vivo ge ...... ce from an ammonium challenge.
@en
Adenovirus-mediated in vivo ge ...... ce from an ammonium challenge.
@nl
P1433
P1476
P2093
P2860
P2888
P304
P31
P356
P407
P433
P478
P577
P6179
P698
P921
P356
P1433
P1476
Adenovirus-mediated in vivo ge ...... ce from an ammonium challenge.
@en
P2093
P2860
P2888
P304
P31
P356
10.1203/00006450-199704000-00012
P407
P433
P478
P577
P6179
P698
P921